S5394 The Chameleon Effect: Distinguishing Drug Induced Liver Injury From Hepatitis C in Hurler Syndrome

Introduction: Drug-induced liver injury (DILI) can result from medications or herbal supplements, with symptoms ranging from elevated liver enzymes to acute liver failure. Hepatic dysfunction, chronic liver disease, HIV and obesity can predispose you to DILI. This case highlights diagnostic challenges in identifying DILI in a patient with Hurler syndrome and newly diagnosed hepatitis C virus (HCV) infection. Case Description/Methods: A 28-year-old man with Hurler syndrome presented with nausea, vomiting, jaundice, and abdominal pain for 1 week. Physical examination revealed facial dysmorphism, jaundice, and right upper quadrant abdominal pain. Laboratory tests showed elevated liver enzymes (alkaline phosphatase 203 U/L, aspartate aminotransferase 1609 U/L, alanine aminotransferase 2019 U/L, total bilirubin 9.3 mg/dL). His liver enzymes were normal 3 months ago. On probing further, patient reported using amoxicillin for an upper respiratory tract infection 3 weeks prior and taking 8 acetaminophen tablets (500 mg) over 2 days for a headache 2 weeks ago. He occasionally drank hibiscus tea but used no herbal supplements. He was sexually active with males and used barrier contraception infrequently. Viral hepatitis tests showed positive HCV antibodies, indicating HCV infection. Imaging studies ruled out obstructive causes, and alcohol testing was negative, making alcoholic hepatitis unlikely. Given the clinical presentation with recent medication use, DILI was suspected. Both amoxicillin and hibiscus tea can predispose to DILI, especially in the setting of hepatic dysfunction from HCV and Hurler syndrome. The patient was started on N-acetylcysteine (NAC) and steroids for suspected DILI. A liver biopsy confirmed acute cholestatic hepatitis, consistent with DILI. The patient responded well to treatment, with enzymes trending down, and was discharged with oral steroids and advice to avoid certain medications, including hibiscus tea. Discussion: DILI risk factors include genetics, environment, and pre-existing liver disease (e.g., HCV, Hurler syndrome causing hepatocyte vacuolization). Here, amoxicillin alone (or combined with hibiscus tea/acetaminophen), alongside HCV and Hurler syndrome, likely caused DILI. The temporal link between medication start, symptom onset, and subsequent improvement with NAC and steroids supports this. DILI is a leading cause of acute liver failure in Western countries. High clinical suspicion based on medication timing and transaminitis is vital for prompt treatment due to high fatality rates.